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Inborn Errors of Immunity and Cytokine Storm Syndromes

Inborn errors of immunity (IEI) are a diverse and growing category of more than 430 chronic disorders that share susceptibilities to infections. Whether the result of a genetic lesion that causes defective granule-dependent cytotoxicity, excessive lymphoproliferation, or an overwhelming infection represents a unique antigenic challenge, IEIs can ...
Whitney, Reid, Neil, Romberg
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Other Immunomodulatory Treatment for Cytokine Storm Syndromes

Cytokine storm syndromes (CSS) include different entities such as macrophage activation syndrome, primary and secondary hemophagocytic lymphohistiocytosis (HLH), and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. An effective management strategy is critical in CSS.
ÖZEN, SEZA, BATU AKAL, EZGİ DENİZ
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Genetics of Acquired Cytokine Storm Syndromes

2019
Secondary hemophagocytic lymphohistiocytosis (sHLH) has historically been defined as a cytokine storm syndrome (CSS) occurring in the setting of triggers leading to strong immunological activation, without any known genetic predilection. However, recent studies have suggested that existing underlying genetic factors may synergize with particular ...
Grant S. Schulert, Kejian Zhang
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Parasitic and Fungal Triggers of Cytokine Storm Syndrome

Infections caused by parasites and fungi can trigger the cytokine storm syndrome (CSS). These infections causing CSS can occur together with acquired immunodeficiencies, lymphomas, the use of immunosuppressive medications, transplant recipients, cancer, autoinflammatory, and autoimmune diseases or less frequently in healthy individuals.
Gary, Sterba, Yonit, Sterba
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Zoonotic Bacterial Infections Triggering Cytokine Storm Syndrome

2019
Zoonotic infections can result in life-threatening complications that can manifest with hemophagocytic lymphohistiocytosis (HLH)/cytokine storm syndrome (CSS). Bacteria constitute the largest group of zoonotic infection-related HLH cases. The growing list of zoonotic bacterial infections associated with HLH/CSS include Brucella spp., Rickettsia spp ...
Zaher K, Otrock, Charles S, Eby
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Murine Models of Familial Cytokine Storm Syndromes

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory disease caused by mutations in effectors and regulators of cytotoxicity in cytotoxic T cells (CTL) and natural killer (NK) cells. The complexity of the immune system means that in vivo models are needed to efficiently study diseases like HLH.
Volkmer, Benjamin   +3 more
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Genetics of Acquired Cytokine Storm Syndromes

Secondary hemophagocytic lymphohistiocytosis (sHLH) has historically been defined as a cytokine storm syndrome (CSS) occurring in the setting of triggers leading to strong and dysregulated immunological activation, without known genetic predilection.
Grant S, Schulert, Kejian, Zhang
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Cytokine Storm Syndromes Associated with Epstein–Barr Virus

2019
Epstein-Barr virus (EBV) is a ubiquitous and predominantly B cell tropic virus. One of the most common viruses to infect humans, EBV, is best known as the causative agent of infectious mononucleosis (IM). Although most people experience asymptomatic infection, EBV is a potent immune stimulus and as such it elicits robust proliferation and activation of
Katherine, Verbist, Kim E, Nichols
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Monocytic Phagocytes in the Immunopathogenesis of Cytokine Storm Syndromes

Cytokine storm syndromes (CSSs) are caused by a dysregulated host immune response to an inciting systemic inflammatory trigger. This maladaptive and harmful immune response culminates in collateral damage to host tissues resulting in life-threatening multisystem organ failure.
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Immunology of Cytokine Storm Syndromes: Natural Killer Cells

Natural killer (NK) cells are innate immune lymphocytes that rapidly produce cytokines upon activation and kill target cells. NK cells have been of particular interest in primary hemophagocytic lymphohistiocytosis (pHLH) since all of the genetic defects associated with this disorder cause diminished cytotoxic capacity of NK cells and T lymphocytes, and
Anthony R, French   +2 more
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